A Case of Neurocutaneous Melanosis in a Child with Congenital Giant Nevi

ABSTRACT

Patients with giant congenital melanocytic nevi may grow up to have melanotic tumors characterized by central nervous system involvement, termed neucutaneous melanosis. Neurocutaneous melanosis is a rare but a significant congenital syndrome characterized by large or multiple congenital melanocytic nevi and benign or malignant pigment cell tumors of the leptomeninges. The prognosis is extremely poor for symptomatic patients, even in the absence of malignant melanoma. We experienced a case of a neurocutaneous melanosis with congenital giant hairy nevi, who grew up to have progressive leptomeningeal melanomatosis and whose neurologic conditions were rapidly deteriorated to death. So we report the case with a brief review of related literature.